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N. Stuebiger, C.M. E. Deuter, I. Guenaydin, M. Zierhut, I. Koetter; Therapy With Interferon Alfa In Isolated Ocular Behçet's Disease . Invest. Ophthalmol. Vis. Sci. 2004;45(13):2675.
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Purpose: Behçet's disease (BD), a systemic vasculitis, is diagnosed according the criteria of the "International Study Group of Behçet's Disease". Patients, suffering from a retinal occlusive vasculitis (arteries and veins involved!), which is typical of BD but who show no other signs of BD are most commonly diagnosed as a "retinal vasculitis of unknown origin", irrespective of the fact, that in up to 20% the ocular involvement is the primary manifestation of this disease. Methods: Seven men and one woman received human recombinant Interferon a2a (IFNa2a), that is very effective in ocular BD. These patients suffered from an occlusive retinal vasculitis, which was refractory to other immunosuppressive therapies. The mean age of our patients was 34 years, five patients were of German origin and 3 patients Mediterraneans. Five patients were HLA B51 positive. Former therapies (Cyclosporin A, Azathioprine, Mycophenolate mofetil, Aciclo– and Ganciclovir, and Steroids) had been ineffective. Initially IFNa2a was applied at a dose of 6 MioIU/day and dose reduction was performed according to a decision tree. Activity of ocular changes was evaluated by the Uveitis Scoring System of BenEzra. Results: During IFN–treatment seven of the eight patients (88%) achieved remission of the ocular manifestations. Due to a compliance problem only incomplete remission was achieved in one patient. Mean visual acuity rose from 0.62 before therapy to 0.81 six month after initiation of IFN. The mean posterior uveitis score fell from 4.3 to 1.8 after two weeks of therapy and improved to 0.2 after one year. After a mean observation period of 36 ± 8 months seven patients are in complete remission and 5 patients are off treatment and free of relapses. Interestingly, 4 of our 8 patients later developed typical BD symptoms during dose reduction of IFN–treatment, thus diagnosis of complete BD could be made. Conclusions: Especially in patients, in whom despite typical ocular BD changes (retinal occlusive vasculitis of arteries and veins), the diagnosis of complete BD according the criteria could not be made, an "isolated ocular BD" can be discussed, especially when they are HLA–B51 positive.The diagnosis of BD was confirmed by efficacy of IFN treatment and the development of complete BD in 50% of the patients with primarily isolated ocular BD.
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